The Cure Page 5
“I believe that Dr. Abbott told you that molecular probes of the brain of the Baltimore gene therapy patient showed extensive involvement of her medial temporal lobes and little to no involvement of other brain areas. The clinical manifestations of our patient’s syndrome are wholly compatible with involvement of his medial temporal lobes. These areas are generally the first parts of the brain to become involved in Alzheimer’s disease. The medial temporal lobes are responsible for significant aspects of long-term memory storage and retrieval. Dr. Abbott is going to explain this further then I’ll demonstrate what our patient can and can’t do.”
Jamie’s head nodded inside his bulky helmet. “We all possess short-term and long-term memories,” he said. “Short-term memory is the ability to recall a small amount of information for a short amount of time—a phone number, for example, for the length of time it takes to dial. The prefrontal cortex located in the frontal lobes is responsible for short-term memory. We usually test this with digit or object recall, but given his language difficulties, the best we can do is this simple experiment. Carrie?”
“It took a while to teach him to do what we wanted,” she said, “but he can do it now.”
She fumbled at his upper-body restraints through her thick gloves until Soulandros was freed. He lifted his arms to look at his hands but otherwise remained quiet in bed. There was a bowl of plucked grapes on his bedside table. She picked up one of them and showed it to him. He followed it closely with his eyes.
“Okay,” she said, “where is the grape?”
She transferred the grape from her right hand to her left and made two fists. She waited several seconds then presented her fists.
Soulandros immediately pointed to her left hand and seized the grape when she opened her hand.
“That’s good,” she said while he chewed. “Let’s do it again.”
After three successful tests, Jamie said, “So, it appears that his short-term memory and thus the memory-based function of his prefrontal cortices are intact. Now let’s look at his long-term memory. Broadly speaking, there are two categories of long-term memory: declarative and procedural. Declarative memories reside in the medial temporal lobes. Our declarative memories are the things we know—facts, events, people, places, objects, all sorts of data. There are two types of declarative memories. Semantic memories are things we learn that don’t relate to personal experience—book learning, for example. Then there are episodic memories, memories that relate to things we have personally experienced—going to a wedding, seeing a parade, things like that.
“Procedural memories are quite different. They are stored in different parts of the brain, namely the cerebellum, the frontal lobes, and the basal ganglia. Procedural memories help us do the tasks that we’ve already learned. We can perform them at any time, automatically, without thinking about them consciously—things like driving a car, playing the piano, dancing. We’re going to look at the patient’s declarative and procedural skills and deficits now, but before we do, we’ve got to speak a little bit about language. Memory and language are linked. A component of our declarative memory is the storage of the meaning of words and their associations—our lexicon. Like other declarative memories, these language-based memories are stored in the temporal lobes. The other component of language is our mental grammar, the rules we’ve learned that allow us to put words together in sequence. These grammatical memories are stored in the frontal lobes and are, I believe, uninvolved in our patient. Okay, lesson over. Carrie?”
Soulandros had been watching Carrie’s face through her acrylic visor. Every time he started to get agitated, she smiled at him and this seemed to be calming.
She gestured around the room and said, “Where are you?”
He followed her arm and replied, “My-my-my-my—”
“Okay, who am I?”
“No-no-no.”
She had some objects laid out on a table, picked up a spoon and said, “What is this?”
He took it in his right hand, smelled it, and said, “I-I-I—”
“What is this?” she said again.
He said nothing.
She took the spoon back and handed him a small sealed tub of peaches.
“What is this?”
He took the tub and sniffed it. “Ta-na-na—”
“Is this food?” she asked.
He stared blankly.
She took the tub from his hand. It was no easy chore peeling off the plastic film with a gloved hand, but she managed. She gave him the tub and the spoon at the same time, and he held one in each hand. After sniffing the peaches, he began spooning the fruit into his mouth until every morsel and every drop of juice was consumed.
Jamie said, “He didn’t know or couldn’t say what the spoon was called—a declarative memory problem, but he instinctively knew what it was for and how to use it—a procedural memory skill.”
Carrie picked up a pen. “Two hours ago, he could not name this item,” she said, holding it up to the camera. “We’ve been working on it.” She turned to the bed. “What is this?”
Soulandros tensed his mouth. She repeated the question. He said, “P. Pen.”
“Very good!” Carrie said. “Pen. He didn’t know his name earlier and we’ve also been working on that too. What is your name?”
His mouth moved but nothing came out.
“What is your name?”
There was nothing.
This time she prompted him, “An—”
“An-dy.”
“Very good! Andy!” she said. “Now let’s see if he knows what to do with the pen. We haven’t tried this yet.”
She gave him the pen and put a piece of paper on a tray. Soulandros held the pen in his fist and did nothing. Carrie reached over, took the pen from him and began doodling on the sheet of paper. Then she handed it back. This time he imitated her, holding the pen with only three fingers and drawing a single line on the page. Then he blinked a few times. His hand began moving rapidly.
“Very good, Andy!” she said, taking the sheet and holding it up, revealing a rough signature scrawl.
“This isn’t all that surprising,” Jamie said. “Writing a signature is an automatic kind of action that doesn’t require the conscious retrieval of stored memory.”
Soulandros began squeezing his legs together, grimacing, and touching his groin over his hospital gown.
“Do you want to pee?” Carrie asked him. “He showed this behavior earlier.”
He grunted and when she released his lower restraints, he immediately climbed out of bed and began moving toward the toilet. He disappeared from view until the audience heard the sound of urination and flushing. Carrie went inside the bathroom and came out, gently guiding him by the shoulder until he was back in bed.
“Toileting is another automatic function,” Jamie said, “and the fact that it’s intact here is another sign that his problems are purely in his declarative memory. Okay, before I get out of this suit, let me summarize what we have. This assessment is preliminary and based on only one patient, but I think we can make some generalizations. His syndrome shows similarities but also significant differences to two comparable processes, Alzheimer’s disease and retrograde amnesia. Like patients with severe Alzheimer’s, he has seemingly lost substantial declarative memory retrieval and most of his language abilities, but unlike severe Alzheimer’s, his onset was acute, he’s not bedridden and incontinent, and he’s completely alert. Also, unlike severe Alzheimer’s, where new learning is essentially non-existent, he seems to have the ability to, at a minimum, relearn some things like his own name and the word for pen.
“Patients with retrograde amnesia develop their conditions suddenly, as a result of trauma, a brain bleed, or a severe psychological stress. They may not remember who they are or how they arrived at a particular location, but they generally have fluent language skills and an intact semantic memory. What they lose is their episodic memory, those things specific to their own experiences. Our patient has, I beli
eve, a unique syndrome that has not previously been described. It seems to be compatible with these altered CREB-like factors I described to you earlier, blocking, on a molecular level, his memory-retrieval gates.
“At this time, it’s impossible to say whether other patients with the syndrome will be affected to a greater or lesser extent. It’s also impossible to say how long the syndrome will last without some kind of therapy. This new virus appears to be self-replicating, which means that there will probably be a continuous production of the altered CREBs, and thus a continuing blockade of memory retrieval. That’s not a good fact. However, the ability to relearn things suggests that there are some regions of the temporal lobes that can store and retrieve new memories despite the presence of these altered CREBs. That is a positive.”
When Jamie returned to the small conference room and rejoined the meeting, a heated discussion about public health measures was working its way around the auditorium. Dr. Hansen, the head of the CDC Global Rapid Response Team, was moderating from the podium. Jamie listened as opinions were batted about whether hospitals should remain open or closed, whether the public should be advised to shelter in place, whether schools and day-care facilities should be closed, whether surgical masks should be made widely available. One of the few things that was universally agreed upon was that doxycycline needed to be administered widely, perhaps to the entire population of the United States. Someone from the CDC was dispatched to make calls about domestic supplies of the antibiotic.
Hansen told the audience that among the highest priorities for the CDC was to get a handle on the risk of Febrile Amnesia Syndrome following the exposure to an infected person, and to get definitive data on the upper bounds of the incubation period after an exposure. Knowing both would inform quarantine decisions.
“Is someone safe after four days?” Hansen asked. “Five? Six? Also, how long is an FAS patient contagious? Only as long as they’re coughing? Longer? Are their bodily fluids infectious? We’re going to need to know a lot more to give good answers to the public. But even with incomplete data, it’s a certainty that the White House is going to want us to be putting out a statement today, probably later this morning.”
The staffer making calls about the nationwide inventories of doxycycline came back in and began talking about what he had learned. Just then Jamie’s mobile began vibrating. When he saw it was from Colin Pettigrew, he muted his microphone and turned away from the camera.
“Colin, did you reach Steadman?”
As Pettigrew talked, Jamie lost track of time. When he hung up and glanced at the timer on the phone, he was shocked the call had lasted only two minutes.
He unmuted the microphone and turned toward the camera.
“I’m sorry to interrupt,” he said loudly, causing Hansen to look over his shoulder toward the screen. “I’ve just gotten a call from Baltimore. Dr. Steadman was found this morning. He’s dead. It was a suicide. I was also informed that the head of Steadman’s lab was located in Montana where he was on vacation. He has reported that unknown to all their collaborators and the study safety committee, they had been having technical difficulties maintaining the stability of the gene therapy virus with the suicide gene in place. About six months ago, to keep the project on its timeline, Steadman ordered him to omit the suicide gene and keep quiet about it.”
Jamie heard Hansen shout, “What?”
Jamie swallowed and said, “I’m afraid we have no fail-safe. We have no known weapon against this.”
9
The air was heavy with antiseptics and gloom.
No one voted to make Jamie their leader, but everyone who was in quarantine looked to him for answers. He had few on offer.
He didn’t hide what he knew. Throughout the previous night, every time he or Carrie Bowman emerged from Andy Soulandros’s isolation room, he was asked if the therapy had kicked in. Soulandros’s brother, Dave, was especially fretful, compulsively doing Sudoku puzzles on his phone to monitor his own mental status. When Jamie wrapped up the CDC meeting, he assembled the health care personnel and somberly told them about the ineffectiveness of doxycycline. Then he repeated the message to the patients and family members swept up in the drama of the previous night.
“How long till we know if we’re in the clear?” a woman asked. She had come in for an injection for her migraines and had been in the waiting room seated next to Dave Soulandros.
“It’s hard to say,” he said. “A couple of days maybe. It’s only a guess at this point.”
“My husband and kids are out there,” she said.
“You don’t want to infect them,” he said.
“I can wear a mask and gloves. They can’t keep us, can they?”
According to the public health officials, they could. By mid-morning, a mandatory quarantine order had been slapped in place at MGH and other Massachusetts hospitals where people had been exposed to suspected cases of FAS. Massachusetts wasn’t alone. A blizzard of social media posts suggested that state and local public health departments were taking similar actions around the country.
The CDC put out a press release at noon, describing the new syndrome and advising people to remain at home until contagion data became available. Schools, colleges, and daycare facilities were urged to close temporarily. Hospitals, at their discretion, would be turning away patients who did not have major medical emergencies. People were advised to wear masks if they had to go out to buy emergency supplies of food and medicine.
Almost immediately, there was a run on cash machines, supermarkets, and pharmacies. Shelves emptied. Sidewalk entrepreneurs appeared, selling surgical masks for five dollars and little bottles of hand sanitizer for twenty.
The White House urged calm and put the Secretary of Homeland Security in front of the cameras in the Briefing Room. He had little of substance to add and failed to inspire confidence. The Surgeon General was summoned to the podium. She did marginally better.
There was a TV at the nurses’ station in the Biocontainment Unit and people gathered around it, watching reporters in masks pointing up toward their windows. Others lounged on their cots, staring at their phones, talking to friends and family, or following events unfolding on social media.
The hospital cafeteria was leaving meals at one of the emergency-exit stairwells, while a floor below, a masked security guard watched the stairwell to make sure there were no runners when the alarm was turned off to collect the food.
As the afternoon turned to evening, Jamie read the CDC guidance documents and watched the news with a mounting sense of dread. There were bound to be more cases; the question was how many? He had been invited onto a CDC working group with a briefing planned for four in the afternoon and he’d get an update then. Gaping holes in the public health guidance were stoking concern, bordering on panic. What were people supposed to do if a loved one came down with FAS? Care for them? Abandon them? And what was going to happen to someone who was alone when stricken? Jamie could imagine the crushing, existential fear of sudden and complete amnesia. Would these poor souls even be able to manage to find food and water inside their own homes?
He retreated to the on-call room and tried to reach Emma again. He had last talked to her at mid-morning when she had sounded bored and unfazed, begging off to make a coffee. He thought he heard someone else in the kitchen, but she swore she was on her own. Since then, he tried calling every hour or so, but she stopped picking up on the landline or on her mobile. Since Emma first sailed into her rebellious years, he had spent a lot of time bubbling inside a stewpot of anger and apprehension, but this had to be a new low.
“Pick up the damn phone,” he muttered.
*
The girls took the Green Line from Brookline to Prudential Center, and when they arrived at eleven-thirty, the mall was fairly busy. Emma didn’t have a lot of money to spend and only purchased some makeup. Kyra wanted to get a top and the two of them wandered from store to store until Kyra saw something she liked at the Ralph Lauren store.
“Too much?” Kyra asked.
“It’s your money.”
“Maybe I’ll pass.”
Kyra bought an inexpensive scarf and the two of them went for lunch at the Cheesecake Factory. One of the waiters, a young guy, swapped tables with a waitress so he could serve a couple of cute girls. They liked his frizzy-haired looks, and arm tattoos, and giggled their way through ordering.
“When do you have to get back?” Kyra asked.
“I don’t. Why?”
“Your retarded dog? Remember him?”
She hadn’t waved off Maria. “The cleaning lady’s going to walk him. And Rommy’s not retarded.”
“You want to see my scarf?” Kyra asked Emma.
“I just saw it.”
She pushed the bag over with a foot. “Have another look.”
Emma took the bait. Under the scarf was the expensive, silk camisole.
“Oh my fucking God,” Emma gasped. “You didn’t.”
“I did.”
“If you got caught your mother would crucify you.”
“I didn’t get caught, did I?”
At first, they didn’t notice the diners around them looking at their phones, hastily paying their tabs, getting up to leave. When the restaurant was close to empty, Kyra wondered what was up.
The waiter came over and asked if they wanted more soda and said, “Hey, did you hear?”
“Hear what?” Emma said.
“There’s some kind of epidemic thing. The manager just said. That’s why people are bugging out.”